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Fragile X syndrome (FXS) is caused by hypermethylation of the FMR1 promoter due to the full mutation expansion (full mutation [FM]: CGG ≥ 200 repeats) and silencing of FMR1. Assessment of mosaicism for active-unmethylated alleles has prognostic utility. This study examined relationships between FMR1 methylation in different tissues with FMR1 messenger ribonucleic acid (mRNA) and intellectual functioning in 87 males with FXS (1.89–43.17 years of age). Methylation sensitive Southern blot (mSB) and Methylation Specific-Quantitative Melt Aanalysis (MS-QMA) were used to examine FMR1 methylation. FMR1 mRNA levels in blood showed strong relationships with FMR1 methylation assessed using MS-QMA in blood (n = 68; R2 = 0.597; p = 1.4 × 10−10) and buccal epithelial cells (BEC) (n = 62; R2 = 0.24; p = 0.003), with these measures also showing relationships with intellectual functioning scores (p < 0.01). However, these relationships were not as strong for mSB, with ~40% of males with only FM alleles that were 100% methylated and non-mosaic by mSB, showing methylation mosaicism by MS-QMA. This was confirmed through presence of detectable levels of FMR1 mRNA in blood. In summary, FMR1 methylation levels in blood and BEC examined by MS-QMA were significantly associated with FMR1 mRNA levels and intellectual functioning in males with FXS. These relationships were not as strong for mSB, which underestimated prevalence of mosaicism.

Introduction: Lyme borreliosis (LB) is a zoonosis with worldwide distribution, mainly in the northern hemisphere countries with predominantly cutaneous, articular, cardiac and neuro-psychiatric manifestations. The greater variety of genospecies that cause disease in Europe and the complex laboratory confirmation contribute to the disease underdiagnose in Portugal. Case description: A 17-year-old boy resident in a rural area, with a history of recurrent tonsillitis, initiates cervical pain, odynophagia and fever (39ºC). Blood work showed leukocytosis (11280/uL) and neutrophilia (8569/uL) and CRP 23.6mg/L. Oropharynx antigen screening for SGA and EBV antibodies were negatives but he was discharged with amoxicillin and clavulanic acid. The next day (7th day of disease) he kept fever (38.3°C), initiated a pruritic maculopapular rash on the face, trunk and limbs with palmoplantar involvement and started complaining of thoracic pain that worsened with decubitus. Blood work revaluation indicated increased leukocytosis (14640/uL) and neutrophilia (11040/uL), CRP 142mg/L, VHS 22mm/h. Chest x-ray had a discreet broncho-hilar reinforcement without pleural effusion or cardiomegaly. Electrocardiogram (EKG) in sinus rhythm, with mild infra ST in DIII and aVF, inverted T wave in V1 and V4 and early repolarization pattern in V2-V3. Due to an episode of tachycardia (270 bpm), cardiac markers were accessed and showed troponin 13932pg/mL, CK 436U/L and BNP 373pg/mL, echocardiography showed slight global left ventricular dysfunction and hyperechogenic pericardium without effusion. He was admitted with clindamycin and penicillin and started anti-congestive therapy with carvedilol and enalapril. On the 10th day of disease, of the etiology investigation it was identified by enzyme-linked immunosorbent assay (ELISA) for Borrelia burgdorferi s.l. IgM antibodies positive (53 UA/mL), IgG antibodies negative with confirmatory test-immunoblot IgM positive with bands OspC Bg, p39, p41. Antibiotic therapy was changed to ceftriaxone and doxycycline which he complied with for 8 and 7 days, respectively. On the 20th day of disease cardiac magnetic resonance showed active inflammatory process in the myocardium, but undilated ventricles with preserved global systolic function. Progressive improvement of cardiac markers was noticed, currently with good ventricular function and at the last observation he was asymptomatic from the cardiovascular point of view. Conclusions: LB with myocarditis is a rarity in pediatrics and diagnosis requires a high index of suspicion. When LB cardiac involvement is readily recognized and treated serious complications can be avoided N/A

Los repetidos episodios de contaminación de aguas superficiales y subterráneas origi­nados por herbicidas utilizados en el cultivo del olivar son motivo de una gran preocupa­ción en muchas regiones mediterráneas. El hecho de que la mayoría de las formulacio­nes comerciales de herbicidas contengan las materias activas en una forma inmediata­mente disponible acentúa el riesgo de pérdi­das por transporte de los herbicidas que se aplican al olivar y, por consiguiente, el ries­go de contaminación de aguas adyacentes. En este trabajo se presenta la preparación de formulaciones de liberación lenta de tres herbicidas (diurón, terbutilazina y MCPA), muy utilizados en el control de malas hier­bas en el olivar, a partir del soporte de los mismos en una montmorillonita modificada con cationes hexadeciltrimetilamonio. Pos­teriormente, se ha evaluado la capacidad de las formulaciones preparadas de reducir la velocidad de liberación de los herbicidas en medio acuoso y de retrasar su lixiviación a través de columnas de suelo, en compara­ción a formulaciones comerciales conven­cionales de los herbicidas. Los resultados sugieren la utilidad de las formulaciones desarrolladas para reducir las pérdidas por transporte de los herbicidas estudiados tras su aplicación a suelos de olivar., Revista de Ciências Agrárias, vol. 33 n.º 1 (2010)

Pancreatic enzyme replacement therapy (PERT) is indicated in case of clinically relevant exocrine pancreatic insufficiency (EPI). Clinical trials addressing PERT have used the coefficient of fat absorption (CFA) to define EPI but this test is cumbersome to perform. Our aim was to compare two easier-to-perform tests to detect clinically relevant EPI: Fecal Elastase-1 (FE-1) and 13 C-Mixed Triglyceride Breath Test (TGBT).

INTRODUCTION: Hypoplastic left heart syndrome (HLHS) is a major cause of cardiac death during the first week of life. The hybrid approach is a reliable, reproducible treatment option for patients with HLHS. Herein we report our results using this approach, focusing on its efficacy, safety and late outcome. METHODS: We reviewed prospectively collected data on patients treated for HLHS using a hybrid approach between July 2007 and September 2014. RESULTS: Nine patients had a stage 1 hybrid procedure, with seven undergoing a comprehensive stage 2 procedure. One patient completed the Fontan procedure. Five patients underwent balloon atrial septostomy after the hybrid procedure; in three patients, a stent was placed across the atrial septum. There were three deaths: two early after the hybrid procedure and one early after stage two palliation. Overall survival was 66%. CONCLUSIONS: In our single-center series, the hybrid approach for HLHS yields intermediate results comparable to those of the Norwood strategy. The existence of dedicated teams for the diagnosis and management of these patients, preferably in high-volume centers, is of major importance in this condition.

Ground and surface water contamination by herbicides commonly applied to olive groves is a matter of current concern in Mediterranean regions. The fact that most commercial herbicide formulations in cur­rent use contain the herbicide in an immedi­ately available form that is readily released into the environment exacerbates the risk of ground and surface water contamination by rapid herbicide transport losses. In this work, we prepared slow release formula­tions of three herbicides (diuron, terbuthy­lazine, and MCPA) widely applied to olive groves by preadsorbing the herbicides on hexadecyltrimethylammonium-modified montmorillonite. Then, we evaluated the ability of the organoclay-based formulations to slow the release of the herbicides into wa­ter and to reduce their leaching through soil columns, as compared to conventional commercial formulations containing the herbicides in a readily available form. The results indicated that organoclay-based for­mulations can be useful to reduce the mobil­ity of the assayed herbicides after applica­tion to soils such as those typically used for olive growing in Mediterranean regions., Los repetidos episodios de contaminación de aguas superficiales y subterráneas origi­nados por herbicidas utilizados en el cultivo del olivar son motivo de una gran preocupa­ción en muchas regiones mediterráneas. El hecho de que la mayoría de las formulacio­nes comerciales de herbicidas contengan las materias activas en una forma inmediata­mente disponible acentúa el riesgo de pérdi­das por transporte de los herbicidas que se aplican al olivar y, por consiguiente, el ries­go de contaminación de aguas adyacentes. En este trabajo se presenta la preparación de formulaciones de liberación lenta de tres herbicidas (diurón, terbutilazina y MCPA), muy utilizados en el control de malas hier­bas en el olivar, a partir del soporte de los mismos en una montmorillonita modificada con cationes hexadeciltrimetilamonio. Pos­teriormente, se ha evaluado la capacidad de las formulaciones preparadas de reducir la velocidad de liberación de los herbicidas en medio acuoso y de retrasar su lixiviación a través de columnas de suelo, en compara­ción a formulaciones comerciales conven­cionales de los herbicidas. Los resultados sugieren la utilidad de las formulaciones desarrolladas para reducir las pérdidas por transporte de los herbicidas estudiados tras su aplicación a suelos de olivar.

Submitted by Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2013-04-12T16:13:30Z No. of bitstreams: 1 RPC 2010 1839.pdf: 172485 bytes, checksum: 69308c3c70eeb7915497380a204f8002 (MD5) Made available in DSpace on 2013-04-12T16:13:30Z (GMT). No. of bitstreams: 1 RPC 2010 1839.pdf: 172485 bytes, checksum: 69308c3c70eeb7915497380a204f8002 (MD5) Previous issue date: 2010

INTRODUCTION: Atrial septal defects (ASD) are among the most common congenital anomalies and account for 10% of congenital heart disease in the pediatric age-group and 30% in adults. Closure is indicated when there is evidence of hemodynamic significance or after a paradoxical embolic event. Ten years ago, percutaneous closure became the treatment of choice in our center for all patients with a clear indication and favorable anatomy. In this paper we report the experience of this first decade. OBJECTIVE: To assess the short- and long-term results of our ten-year experience with percutaneous closure of atrial septal defects. METHODS: We studied retrospectively all patients with ASD treated with a percutaneous approach between November 1998 and December 2008. The pediatric age-group consisted of patients younger than 19 years old. Demographic data, clinical indications, minor and major complication rates, success rate and long-term outcome were assessed. RESULTS: In the first ten years of experience 510 patients, of whom 166 were in the pediatric group, were treated in our center by a team of adult and pediatric cardiologists. The overall success rate of the procedure was 98% (97.5% in ASD and 99.5% in patent foramen ovale (PFO). The minor complication rate was 3% (3.4% in ASD and 2% in PFO). The most frequent complication was supraventricular tachycardia. The major complication rate was 1.2% (0.6% in ASD and 2% in PFO). Two patients developed cardiac tamponade due to hemopericardium that was resolved by pericardiocentesis, without need for surgery. One patient had an arterial pseudoaneurysm corrected by vascular surgery. There was no device embolization and no need for urgent surgery in this population. During follow-up two patients had recurrence of ischemic stroke, one had a transient ischemic attack and another had a hemorrhagic stroke. Mortality was 0.6% (0.6% in ASD and 0.5% in PFO). There were no in-hospital deaths. During follow-up there were two deaths, both in the adult group. DISCUSSION AND CONCLUSION: In this population the success rate was high and most of the complications were minor. The results of this collaboration between adult and pediatric cardiologists in the first ten years of activity confirm the safety and efficacy of percutaneous closure of septal defects, when there is careful patient selection and a standardized technique.

Os doentes com cardiopatias submetidos a intervenção cirúrgica com implantação de condutos entre o ventrículo direito e a artéria pulmonar (VD-AP) podem desenvolver estenose e/ou insuficiência pulmonares ao longo do tempo. Nestas situações, associadas a arritmias cardíacas, disfunção ventricular direita e morte súbita, a re-intervenção cirúrgica permanece um desafio pela complexidade, mortalidade e morbilidade significativas. A implantação percutânea de válvulas pulmonares (IPVP), recentemente desenvolvida, representa uma abordagem alternativa para estes doentes. Objectivo: Reportar a experiência inicial com a IPVP, analisando o seu impacto no manejo destes doentes. Material e Métodos: Avaliação prospectiva dos dados clínicos, ecocardiográficos, da ressonância magnética, hemodinâmicos e angiográficos dos doentes submetidos a IPVP. Resultados: Seis doentes em classe funcional igual ou superior a II com disfunção do conduto VD-AP foram submetidos a IPVP. Tinham todos evidência de insuficiência e 5/6 de estenose. O procedimento consistiu na implantação de stents não cobertos no conduto para reforçar a sua rigidez e prevenir fracturas dos stents valvulados pulmonares Melody ® (Medtronic), que foram em seguida implantados com sucesso em todos. Obteve-se uma redução da pressão ventricular direita (94±27 para 44±7mmHg), da relação entre a pressão ventricular direita e esquerda (94±27 para 44±7%), do gradiente do conduto (65±28 para 11±4mmHg) e ausência de regurgitação pulmonar. O procedimento teve uma duração mediana de 180 minutos e decorreu sem complicações major. Os doentes tiveram alta em mediana dois dias após o procedimento. Na última avaliação, em mediana 7,8 meses após o procedimento, os doentes encontramse em classe funcional I (5) ou II (1), sem evidência não invasiva de disfunção do conduto. Conclusões: Os nossos resultados acompanham a experiência actual com esta modalidade terapêutica, com excelentes resultados nestes grupo de doentes complexos. A implementação da IPVP em doentes com cardiopatia congénita que requerem re-intervenção no tracto de saída do ventrículo direito tem-se revelado uma técnica promissora, embora complexa, alternativa a mais uma intervenção cirúrgica.

Submitted by Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2011-05-03T16:25:54Z No. of bitstreams: 1 RPC 2010 767.pdf: 171377 bytes, checksum: 5e1973d6096deed5629f1187d34580a4 (MD5) Made available in DSpace on 2011-05-03T16:25:54Z (GMT). No. of bitstreams: 1 RPC 2010 767.pdf: 171377 bytes, checksum: 5e1973d6096deed5629f1187d34580a4 (MD5) Previous issue date: 2010

Introdução: Os tumores cardíacos primários podem ser benignos ou malignos, ter origem no endocárdio, miocárdio ou pericárdio. São raros em crianças, com uma prevalência descrita em séries de autópsia de 0,0017 a 0,28%; a maioria são benignos, representando os malignos cerca de 10% do total. Material e Métodos: Com o objectivo de avaliar a apresentação clínica e a evolução das crianças assistidas num centro de Cardiologia Pediátrica com este diagnóstico, procedeu-se à revisão retrospectiva dos processos clínicos dos doentes com tumores cardíacos primários observados nos últimos 17 anos (1989-2006). Determinou-se a forma de apresentação, diagnóstico morfológico, complicações e evolução ao longo do período de seguimento. Resultados: Identificaram-se 12 doentes, cuja idade média à data do diagnóstico foi de 23 meses. Em dois casos o diagnóstico foi pré-natal. A causa mais frequente de referenciação foi a esclerose tuberosa, seguida de sopro cardíaco. O ecocardiograma e a ressonância magnética permitiram o diagnóstico em todos. Todos os doentes realizaram electrocardiograma e registo Holter e o achado mais frequente foram as alterações inespecíficas da repolarização. O tumor mais frequente foi o rabdomioma (67%), na maioria associado a esclerose tuberosa; seguido do fibroma em (17%) e do fibroelastoma (8%). Apesar da biópsia ser o exame de eleição para a confirmação diagnóstica, realizou-se apenas em dois doentes. A excisão cirúrgica do tumor foi efectuada num doente (fibroelastoma), por risco de embolia pulmonar. Discussão e Conclusões: Na população estudada o tumor cardíaco mais frequente foi o rabdomioma associado à esclerose tuberosa. A maioria dos doentes não apresentava sintomas cardiovasculares, sendo o seu diagnóstico geralmente efectuado em observações de rotina para esclarecimento de sopro cardíaco ou devido ao diagnóstico de esclerose tuberosa. O exame histológico justifica-se apenas nos tumores mais raros.

Submitted by Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2013-04-09T15:13:24Z No. of bitstreams: 1 RPC 2009 929.pdf: 238429 bytes, checksum: 4c1c8c5adb2ff19d6212d9887076195a (MD5) Made available in DSpace on 2013-04-09T15:13:24Z (GMT). No. of bitstreams: 1 RPC 2009 929.pdf: 238429 bytes, checksum: 4c1c8c5adb2ff19d6212d9887076195a (MD5) Previous issue date: 2009

Submitted by Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2011-04-28T15:55:42Z No. of bitstreams: 1 RPC 2009 799.pdf: 166603 bytes, checksum: 5a34cb78efa2ba4848b638073c0be5b3 (MD5) Made available in DSpace on 2011-04-28T15:55:42Z (GMT). No. of bitstreams: 1 RPC 2009 799.pdf: 166603 bytes, checksum: 5a34cb78efa2ba4848b638073c0be5b3 (MD5) Previous issue date: 2009

INTRODUCTION: Chylothorax is a rare but serious postoperative condition in children with congenital heart disease. Conventional medical treatment consists of specific long-term dietary modification, and surgical reintervention, such as lymphatic duct ligation, may be indicated in refractory cases. In recent years, an additional conservative treatment, octreotide, a synthetic analog of somatostatin, has been used in management of congenital and postoperative chylothorax. METHODS: The objective of this work was to analyze the efficacy and safety of this treatment for chylothorax after congenital heart surgery. We reviewed the records of sixteen patients with chylothorax after surgery for congenital heart disease between January 1999 and December 2007, and collected the following data: demographic information; type of surgical procedure; onset, duration and management of chylothorax and treatment; and duration of hospital stay. To analyze efficacy we compared these parameters in children receiving conventional treatment only with those receiving octreotide. To analyze safety we compared the adverse effects of both treatments. Octreotide was administered at a dose of 4 to 10 microg/kg/hour, with monitoring of side effects. RESULTS: The incidence of chylothorax in our population was 1.6%. It occurred more often after Glenn and Fontan procedures (8 patients). Octreotide was begun three days after diagnosis of chylothorax and continued for a median of seventeen days (ranging from 4 to 26 days), until complete resolution. Side effects were frequent (in 3 of the 8 patients) but of no clinical relevance. All patients responded to the therapy and there was no indication for further surgical intervention. DISCUSSION AND CONCLUSIONS: Octreotide is safe and effective in the treatment of postoperative chylothorax in children with congenital heart disease. It is a useful adjunctive therapy to the conventional treatment of this complication.

Objetivo: comparar dos pautas de pantoprazol por vía intravenosa en pacientes con hemorragia digestiva alta (HDA) ulcerosa de alto riesgo para presentar persistencia o recidiva hemorrágica. Material y método: se randomizaron los pacientes en dos grupos: grupo 0: tratamiento con bolo de 80 mg i.v. de pantoprazol y perfusión continua a 8 mg/h durante 72 horas; grupo 1: tratamiento con 40 mg i.v. de pantoprazol diarios. Se analizó el porcentaje de persistencia/recidiva hemorrágica entre ambos grupos, requerimientos transfusionales, necesidad de cirugía y mortalidad del episodio hemorrágico. Resultados: se incluyeron 20 pacientes en el grupo 0 y 21 en el grupo 1. No se encontraron diferencias entre ambos grupos en cuanto al sexo, edad, hábito tabáquico, consumo de AINE, presencia de inestabilidad hemodinámica, estigma sobre el nicho ulceroso (Forrest Ia 5 vs. 14,3%, p = 0,322; Forrest Ib 30 vs. 33,3%, p = 0,819; Forrest IIa 60 vs. 50,1%, p = 0,753). El 90% de los pacientes del grupo 0 recibió tratamiento endoscópico vs. el 100% del grupo 1, p = 0,232. El 50% de los pacientes del grupo 0 recibió transfusión vs. el 52,4% del grupo 1, p = 0,879. Dos pacientes (10,5%) del grupo 0 presentaron recidiva hemorrágica vs. 3 pacientes (14,3%) del grupo 1, precisando cirugía 1 paciente de cada grupo y falleciendo 1 paciente del grupo 0. Conclusiones: la inhibición ácida máxima de la secreción ácida gástrica mediante bolo e infusión continua de pantoprazol no ofrece resultados superiores al tratamiento con dosis convencionales en el episodio hemorrágico agudo. Objective: to compare two regimens of pantoprazole administered intravenously in patients with ulcerative gastrointestinal bleeding (UGB), and a high risk of presenting with persitent or recurrent hemorrhage. Material and method: patients were randomized into two groups: group 0 - treatment with a 80 mg bolus of pantoprazole administered intravenously, followed by continuous infusion of 8 mg/h for 72 hours; group 1 - treatment with 40 mg of pantoprazole administered intravenously on a daily basis. The percentage of hemorrhagic persistence/recurrence in both groups was analyzed, as were transfusion requirements, need for surgery, and mortality resulting from the hemorrhagic episode. Results: there were 20 patients in group 0 and 21 in group 1. No differences were found between groups in terms of gender, age, smoking habits, use of NSAIDs, presence of hemodynamic instability or stigmata in ulcer crater (Forrest Ia: 5 vs. 14.3%, p = 0.322; Forrest Ib: 30 vs. 33.3%, p = 0.819; Forrest IIa: 60 vs. 50.1%, p = 0.753). In group 0, 90% of patients received endoscopic treatment, versus 100% in group 1, p = 0.232. In group 0, 50% of patients had a transfusion, as compared to 52.4% in group 1, p = 0.879. In group 0, 2 patients (10.5%) presented with recurrent hemorrhage, versus 3 patients (14.3%) in group 1. Surgery was required by 1 person from each group, and 1 patient in group 0 died. Conclusions: maximum acid inhibition with a bolus and a then a continuous infusion of pantoprazole does not yield better results than treatment with conventional doses in acute hemorrhagic episodes.

Submitted by Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2013-04-05T16:42:34Z No. of bitstreams: 1 RPC 2008 1339.pdf: 374827 bytes, checksum: aed9f9a6e555052bac90cea8878de22d (MD5) Made available in DSpace on 2013-04-05T16:42:34Z (GMT). No. of bitstreams: 1 RPC 2008 1339.pdf: 374827 bytes, checksum: aed9f9a6e555052bac90cea8878de22d (MD5) Previous issue date: 2008

Submitted by Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2011-05-23T16:25:35Z No. of bitstreams: 1 AMP 2008 383.pdf: 111259 bytes, checksum: b548fba7bcdaf2a309edccbfc54aee9d (MD5) Made available in DSpace on 2011-05-23T16:25:35Z (GMT). No. of bitstreams: 1 AMP 2008 383.pdf: 111259 bytes, checksum: b548fba7bcdaf2a309edccbfc54aee9d (MD5) Previous issue date: 2008

Objetivos: analizar la evolución, a lo largo del periodo 1999-2005, de las siguientes variables de los pacientes ingresados por hemorragia digestiva (HD) en una Unidad de Sangrantes: etiología, patología de base, consumo de AINE/anticoagulación y mortalidad. Material y métodos: durante el periodo 1999-2005 se ha estudiado la evolución de las siguientes causas de HD que requirieron ingreso en la Unidad de Sangrantes: ulcus duodenal (UD), ulcus gástrico (UG), hipertensión portal (HPT) y otros. De igual forma se ha analizado la evolución en el porcentaje de enfermos ingresados con patología de base, consumo de fármacos AINE/anticoagulantes y mortalidad. Resultados: se han incluido 1.611 pacientes en el estudio con una edad media de 60,45 años (59,7-61,2), 76,41% hombres (74,3-78,5). La UD fue responsable del 22,20% de episodios (20,2-24,3), la UG del 18,40% (16,6-20,4) y la HPT del 33,60% (31,3-36,0). De forma global realizaban tratamiento con AINE el 34,5% (32,6-37,3), anticoagulación el 7,1% (6,0-8,6), presentaban patología de base el 72,6% (70,4-74,8) y la mortalidad global fue del 6,27% (5,16-7,59). A lo largo del periodo estudiado se constató un aumento de los pacientes con patología de base (p < 0,02) y un descenso en los ingresos por UD (p < 0,04), permaneciendo estables el resto de variables estudiadas. Conclusiones: la UD y UG junto con la HPT suponen el 75% de los ingresos en la Unidad de Sangrantes. A lo largo de los últimos 7 años se constata un descenso de los casos debido a UD y un aumento de pacientes con patología de base, mientras la mortalidad global se mantiene estable. Objectives: to analyze the evolution of the following variables in patients admitted to a Blood Unit for gastrointestinal bleeding throughout 1999-2005: etiology, comorbid diseases, use of NSAIDs/anticoagulants, and mortality. Material and methods: we analyzed the evolution of the following causes of GIB that required admission to the Blood Unit from 1999 to 2005: duodenal ulcer (DU), gastric ulcer (GU), portal hypertension (PHT), and others. We also analyzed changes in the percentage of patients admitted with comorbid disease, use of NSAIDs/anticoagulants, and mortality. Results: 1,611 patients with a mean age of 60.45 years (59.7-61.2) were included in this study; 76.41% were males (74.3-78.5). DU was the cause of bleeding in 22.20% of cases (20.2-24.3), GU in 18.40% of cases (16.6-20.4), and PHT in 33.60% of cases (31.3-36.0). In all, 34.5% (32.6-37.3) of patients were taking NSAIDs, 7.1% (6.0-8.6) were receiving anticoagulant therapy, 72.6% (70.4-74.8) presented with comorbid disease, and overall mortality was 6.27% (5.16-7.59). Throughout the 1999-2005 period there was an increase in the number of patients with comorbid diseases (p < 0.02), and a decrease in cases of DU (p < 0.04), without significant differences in the remaining variables. Conclusions: DU, GU and PHT account for three quarters of admissions to our Blood Unit. Over the last seven years, there has been a decrease in cases due to DU, and an increase in patients with comorbid disease; overall mortality rates have remained stable.

Cavopulmonary connections have been extensively used in the palliation of complex forms of congenital heart disease requiring some form of right heart bypass. We examine the mid term outcomes of pulmonary ventricle bypass operations in a single institution and performed by the same surgical team. POPULATION: Between March 1999 and April 2006, 62 patients underwent pulmonary ventricle bypass operations: bidirectional cavopulmonary anastomosis (Glenn procedure), total cavopulmonary connections (Fontan procedure) and one and a half ventricle correction in two cases. Age at operation averaged three years (range: 0.42-25 years) for the Glenn procedure and seven years (range: 3-14 years) for the Fontan procedure. There were 36 male patients (58%) and 26 female patients (42%). The most common indication for surgery was the single ventricle defect, present in 66% of patients. Associated lesions included: transposition of the great arteries in 16 patients (35.6%), bilateral superior vena cava in four patients (8.9%), situs ambigus in five patients (11%), situs inversus in another patient (2.2%), Ebstein disease in one patient (2.2) and coronary fistula in another patient (2.2%). Sub-aortic stenosis was present in one patient (2.2%). Palliative surgery was performed in all, but three patients (5%), before the Fontan procedure. RESULTS: Thirty two patients underwent bidirectional cavopulmonary anastomosis and thirty patients underwent cavopulmonary connections, total or 2nd stage. Mean cardiopulmonary bypass times were 50.6+/-21.9 minutes for the Glenn procedure and 88.5+/-26.3 minutes for the Fontan procedure. There was no intra-operative mortality, but two patients (3.2% (died in the first month after surgery; one due to failure of the Glenn circuit and sepsis and the other due to a low cardiac output syndrome and multi-organ dysfunction. Mean ventilation time was 5.2+/-1.7 hours for the Glenn operation and 6.2+/-3.2 hours for the Fontan operation. The mean length of stay in ICU was 3.4+/-2.8 days for patients undergoing the Glenn operation and 4.6+/-3.1 days for patients undergoing the Fontan operation and the mean length of hospital stay was 10.6+/-5.8 days for the Glenn operation and 19.1+/-12.6 days for the Fontan operation respectively. The mean follow up time was 4+/-2.1 years (minimum 0 years and maximum seven years), most patients being in NYHA class I. Epicardiac pacemakers were implanted in three patients due to arrhythmias. Two re-operations (6.7%) were needed, both in the same patient, after the Fontan procedure, this patient eventually died a few years after surgery. CONCLUSIONS: The immediate and mid term outcomes of pulmonary ventricle bypass operations can have excellent results. From our point of view there has been an improvement, namely in the use of the extracardiac conduit technique in the 2nd stage of the Fontan operation.

Submitted by Ana Quininha (ana.quininha@chlc.min-saude.pt) on 2013-04-02T11:29:00Z No. of bitstreams: 1 RPC 2006 477.pdf: 56773 bytes, checksum: fde873f7e01dd6135f24716f14f3f39a (MD5) Made available in DSpace on 2013-04-02T11:29:00Z (GMT). No. of bitstreams: 1 RPC 2006 477.pdf: 56773 bytes, checksum: fde873f7e01dd6135f24716f14f3f39a (MD5) Previous issue date: 2006